NYMC Faculty Publications

An Update and Report Failure of Surgical Syndactyly Repair in Harlequin Ichthyosis

Author Type(s)

Faculty

Journal Title

Plastic and Reconstructive Surgery. Global Open

First Page

e4594

Document Type

Article

Publication Date

10-1-2022

Department

Surgery

Abstract

Harlequin ichthyosis (HI) is a rare congenital skin disorder caused by irregular epidermal differentiation. Syndactyly in HI is associated with thick hyperkeratotic skin flexion and angulation deformity of the hand and fingers resulting in limited function of the upper extremity. Traditional syndactyly release is limited as full-thickness skin grafts typically used in reconstruction are composed of diseased skin and require donor sites in a patient predisposed for adverse wound healing. This case report is a follow-up to a previous viewpoint written about digital escharotomies in a newborn with HI and outlines a second and fourth webspace syndactyly release with a dermal substitute. Despite early evidence of adequate release and improved hand function, recurrence of syndactyly was observed within 4 months of surgical release. Our experience described within this case report may suggest the limitations and possible alternatives of surgical release of syndactyly in the HI population.

Link to Full Text

Share

COinS